Results 51 to 60 of about 5,172,961 (263)

Induced pluripotent stem cell (iPSC) lines from two individuals carrying a homozygous (BGUi007-A) and a heterozygous (BGUi006-A) mutation in ELP1 for in vitro modeling of familial dysautonomia

Stem Cell Research, 2021
Familial Dysautonomia (FD) is an autosomal recessive congenital neuropathy affecting the development and function of the peripheral nervous system. FD causing gene is IKBKAP, encoding IkappaB kinase complex-associated protein also named elongator complex
Lior Dor, Tatiana Rabinski, Dor Zlotnik, Michal Shilian, Miguel Weil, Gad D. Vatine   +5 more
doaj   +1 more source

The Little Elongation Complex Functions at Initiation and Elongation Phases of snRNA Gene Transcription [PDF]

Molecular Cell, 2013
The small nuclear RNA (snRNA) genes have been widely used as a model system for understanding transcriptional regulation due to the unique aspects of their promoter structure, selectivity for either RNA polymerase (Pol) II or III, and because of their unique mechanism of termination that is tightly linked with the promoter.
Hu, Deqing, Smith, Edwin R., Garruss, Alexander S., Mohaghegh, Nima, Varberg, Joseph M., Lin, Chengqi, Jackson, Jessica, Gao, Xin, Saraf, Anita, Florens, Laurence, Washburn, Michael P., Eissenberg, Joel C., Shilatifard, Ali   +12 more
openaire   +2 more sources

Reversible Stalling of Transcription Elongation Complexes by High Pressure [PDF]

Biophysical Journal, 1998
We have investigated the effect of high hydrostatic pressure on the stability of RNA polymerase molecules during transcription. RNA polymerase molecules participating in stalled or active ternary transcribing complexes do not dissociate from the template DNA and nascent RNA at pressures up to 180 MPa.
Erijman, L., Clegg, R.
openaire   +3 more sources

Elongated Dihydrogen versus Compressed Dihydride in Osmium Complexes [PDF]

Chemistry – A European Journal, 2017
AbstractSmall modifications on the co‐ligands of complexes containing two coordinated hydrogen atoms can determine the elongated dihydrogen versus compressed dihydride nature of these species and therefore their chemical behavior. 2,6‐diphenylpyridine favors the formation of the osmium(IV) cation [OsH2(C6H4pyPh)(PiPr3)2]+, whereas 2‐phenoxy‐6 ...
Beatriz Eguillor, Miguel A. Esteruelas, Virginia Lezáun, Montserrat Oliván, Enrique Oñate   +4 more
openaire   +3 more sources

Reduction of retinal ganglion cell death in mouse models of familial dysautonomia using AAV-mediated gene therapy and splicing modulators

Scientific Reports, 2023
Familial dysautonomia (FD) is a rare neurodevelopmental and neurodegenerative disease caused by a splicing mutation in the Elongator Acetyltransferase Complex Subunit 1 (ELP1) gene.
Anastasia Schultz, Shun-Yun Cheng, Emily Kirchner, Stephanann Costello, Heini Miettinen, Marta Chaverra, Colin King, Lynn George, Xin Zhao, Jana Narasimhan, Marla Weetall, Susan Slaugenhaupt, Elisabetta Morini, Claudio Punzo, Frances Lefcort   +14 more
doaj   +1 more source

Residual tail twisting in ascidian larvae is stabilized by asymmetric myofibrils that resist bilateral symmetry restoration

FEBS Letters, EarlyView.
Ascidian Ciona larvae initially show strong clockwise tail twisting, which is largely corrected during development. However, a small residual twist remains. This study shows that organized helical myofibrils in tail muscles mechanically stabilize this residual asymmetry, preventing complete restoration of bilateral symmetry and revealing how embryos ...
Yuki S. Kogure, Misaki K. Nishio, Satoru Okuda, Kohji Hotta   +3 more
wiley   +1 more source

The Arabidopsis Elongator Subunit ELP3 and ELP4 Confer Resistance to Bacterial Speck in Tomato

Frontiers in Plant Science, 2018
Although production of tomato (Solanum lycopersicum) is threatened by a number of major diseases worldwide, it has been difficult to identify effective and durable management measures against these diseases.
Juliana A. Pereira, Fahong Yu, Yanping Zhang, Jeffrey B. Jones, Zhonglin Mou   +4 more
doaj   +1 more source

Septin 9 PB domains coordinate centrosome positioning and microtubule acetylation to control epithelial polarity

FEBS Letters, EarlyView.
Septin 9 polybasic domains couple phosphoinositide‐rich membrane binding to centrosome positioning, Golgi organization, and microtubule acetylation to control epithelial polarity. Their loss disrupts this axis, causing centrosome mispositioning, Golgi fragmentation, reduced microtubule acetylation, and polarity inversion via upregulation of the ...
Ting ting Cai, Mohyeddine Omrane, Nassima Benzoubir, Didier Samuel, Ama Gassama‐Diagne   +4 more
wiley   +1 more source

Phosphorylation of Elp1 by Hrr25 is required for elongator-dependent tRNA modification in yeast.

PLoS Genetics, 2015
Elongator is a conserved protein complex comprising six different polypeptides that has been ascribed a wide range of functions, but which is now known to be required for modification of uridine residues in the wobble position of a subset of tRNAs in ...
Wael Abdel-Fattah, Daniel Jablonowski, Rachael Di Santo, Kathrin L Thüring, Viktor Scheidt, Alexander Hammermeister, Sara Ten Have, Mark Helm, Raffael Schaffrath, Michael J R Stark   +9 more
doaj   +1 more source

Structural insights and therapeutic targets in Acinetobacter baumannii capsule biosynthesis

FEBS Letters, EarlyView.
Hypervirulent KL49 A. baumannii's capsular polysaccharide contains the nonulosonic acid 8‐epi‐Leg5,7Ac2, synthesized by epimerization via ElaA, ElaB, and ElaC. Crystal structures of ElaA, ElaB, and ElaC reveal their role in CMP‐Leg5,7Ac2 synthesis and regioselective C8 epimerization.
Woo Cheol Lee, Junho Jeong, Chae Yeong Lee, Hyunjoon Cho, Man Hwan Oh, Je Chul Lee, Jee‐Young Lee, Yangmee Kim   +7 more
wiley   +1 more source